Yuan's team exposed human brain cells to Congo red between 6 and 48 hours after they began
producing huntingtin.
Not exact matches
In March Chris Ross and his colleagues reported that cells bearing mutant
huntingtin could survive if they
produced extra CBP.
The stutters
produce long stretches of the amino acid glutamine in the
huntingtin protein, and the resulting misshapen protein clumps up within neurons, destroying brain cells.
At the same point in the disease process, the scientists found no evidence of impairment in liver cells, which also
produce the mutated
huntingtin protein.
That means a healthy parent whose
huntingtin gene encodes proteins with 35 repeats may
produce a child with 36 repeats.
An approach to treating HD that uses targeted molecules to tell cells not to
produce the harmful
huntingtin protein
R6 / 2 mice have been genetically altered with an abnormal gene that makes them
produce a harmful fragment of the mutant
huntingtin protein
Note: these mice don't
produce toxic mutant
huntingtin protein.
More than 36 CAG repeats in the
huntingtin gene will always lead to HD symptoms, if a person lives long enough, and longer CAG repeats tend to
produce an earlier age of onset.
When a person has too many CAGs in a row near the start of the
huntingtin gene, a harmful «mutant» form of the protein is
produced based on the instructions in the gene.
It turns out that faulty processing of the
huntingtin «recipe»
produces a short, harmful fragment of the
huntingtin protein.
It's the extra-long copy of the
huntingtin gene that makes neurons sick, because it causes them to
produce an extra-long, harmful version of the
huntingtin protein.
That's not what we expect when patients are given
huntingtin - lowering drugs, which might
produce around 50 - 75 % reductions in the mutant and healthy protein.
It's the world's first trial in patients to slow, stop, prevent or reverse HD, using a method that targets the root cause of the disease, a gene
producing a protein called
huntingtin.